Jubilee Editorial Hypertrophic cardiomyopathy

When I first used the expression "cardiomyopathy" in 1956 I did not realise that I had coined a term that would become widely applied. Experience, however, has confirmed its value in describing isolated noncoronary, non-hypertensive, and non-valvar myocardial disease, especially when used with an adjective if the aetiology or morphology is sufficiently clear. Some authorities prefer to restrict its use to disorders of the myocardium of unknown cause; this would exclude hypertrophic cardiomyopathy which in most patients is an inherited disorder. Although the division into hypertrophic and congestive cardiomyopathies has proved helpful, classification remains somewhat unsatisfactory despite many worthy attempts to clarify the position. Unexplained and isolated myocardial hypertrophy was described in the nineteenth century by German pathologists and an obstructive element was recognised in their reference to "conus stenosis". Two patients with massive hypertrophy were reported in 1907 by Schminke who considered that hypertrophy led to further hypertrophy.2 Several papers were published in the 1930s and 1940s under various titles describing isolated myocardial hypertrophy, and Evans's paper on "familial cardiomegaly" in the British Heart Journal was particularly important.3 He described nine patients seen at the London Hospital and emphasised the diagnostic importance of a family history of similar disease, frequent arrhythmias, and a tendency to sudden death. Brock later added another facet to the story in suggesting the possibility that hypertrophic muscle was responsible for obstruction.4 He had observed and was impressed by apparent obstruction of the outflow tract by hypertrophic muscle after pulmonary stenosis had been relieved by valvotomy and he later described apparently important muscular obstruction in the left ventricle. Teare, a forensic pathologist, followed with a description,